Ligneous cervicitis and endometritis: A gynaecological presentation of congenital plasminogen deficiency

Abstract

This is a chronic condition requiring a multidisciplinary approach. There is currently no definitive treatment for the condition, current trials with plasminogen concentrate replacement therapy may provide a promising option for these patients in the future.

A Peculiar Disease of a Young Woman who Wants to Get Pregnant

“…Severe hypoplasminogenemia is associated with ligneous conjunctivitis, but ligneous lesions can also occur in many different mucosal membranes like the cervix and endometrium [ 4 , 5 ]. Despite numerous clinical evaluations, biopsies, and laboratory tests, these kinds of diagnoses remained elusive for many years [ 6 ]. By presenting this case, we want to increase the awareness of this peculiar disease for its proper diagnosis.…”

“…The pathological evaluation of membranes from endocervical curetting showed woody fibrin accumulation, patchy ulceration, and polymorphonuclear leukocyte exudate [ 5 ]. Her menstrual cycles were normal and she had no problems during intercourse [ 6 ]. As plasma-derived plasminogen concentrate (NCT02312180) was not available in Turkey, fresh frozen plasma (FFP) was given both intravenously and directly into the uterine cavity in an off-label way.…”

Congenital plasminogen deficiency with long standing pseudomembranous conjunctival and genital lesions

“…This disease usually appears in childhood, often in a severe manner, leading to complications such as blindness, hydrocephalus, respiratory failure caused by bronchial obstruction, or even death 2 . Patients who reach adulthood usually have a history of pneumonia, cervicitis, conjunctivitis, hoarseness, or sterility 3 . We report on a 60-year-old woman with conjunctival and vulvar pseudomembranous lesions finally diagnosed as congenital plasminogen deficiency.…”

Inflammatory Bowel Disease: A Gastrointestinal Presentation of Congenital Plasminogen Deficiency

“…3 The disordered extracellular fibrin clearance can also affect other mucosal areas such as the female genital tract, oropharynx, upper and lower respiratory tract, renal collecting system, and duodenum. 2,5…”